ANCA-Associated Vasculitis in a Patient Presenting With Rapid Progressive Glomerulonephritis

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare disease distinguished by the presence of circulating ANCA along with inflammation and destruction of primarily small blood vessels. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Overall, AAV occurs more frequently in Caucasian populations with an approximate incidence of 20 per million per year in Europe and North America. This report presents a case of a 70-year-old female with a history of interstitial lung disease who was hospitalized due to markedly reduced renal function and eGFR within the range of end-stage renal disease on admission. The patient tested positive for perinuclear (p)-ANCA, also known as myeloperoxidase (MPO)-ANCA. The patient was subsequently started on hemodialysis and induction therapy of cyclophosphamide and methylprednisolone for glomerulonephritis secondary to p-ANCA vasculitis. The patient was discharged with improved renal function, and she was expected to follow up with nephrology for maintenance therapy to prevent future relapse. This report demonstrates a case of p-ANCA-positive glomerulonephritis treated with cyclophosphamide and methylprednisolone and discusses the current treatment guidelines for glomerulonephritis secondary to p-ANCA vasculitis.